Reducing Postoperative Abdominal Bulge Following Deep Inferior Epigastric Perforator Flap Breast Reconstruction with Onlay Monofilament Poly-4-Hydroxybutyrate Biosynthetic Mesh.

Background The purpose of this study was to evaluate the use of a biosynthetic mesh onlay on reducing postoperative abdominal bulge following deep inferior epigastric perforator (DIEP) flap breast reconstruction. Methods All patients undergoing DIEP reconstructions from January, 2010 to January, 2014 at a tertiary center were reviewed. Patients were divided into two groups for comparison based on whether a biosynthetic mesh onlay (Phasix [monofilament poly-4-hydroxybutyrate], Bard Inc., Warwick, RI) was used for reinforcement of the anterior rectus fascia. Rates of postoperative abdominal bulge were compared between the groups utilizing standard statistical methods. Results During the study period, 319 patients underwent 553 DIEP reconstructions, 160 (50.2%) used mesh and 159 (49.8%) did not (nonmesh). The mean follow-up was 16.4 ± 11.1 months. There was no difference in age (49 ± 9.3 years), current tobacco use, diabetes, or mean body mass index (BMI, 29.4 ± 4.4) between the mesh and nonmesh groups (p > 0.05); however, there was a higher proportion of obese patients (BMI > 30) in the mesh group (45.0 vs. 33.3%; p = 0.03). Abdominal bulge rate following DIEP with mesh was lower than the nonmesh group (0 vs. 5.0%; p = 0.004). In the entire sample, 234 (73.4%) underwent bilateral DIEP and 85 (26.6%) underwent unilateral DIEP. In unilateral DIEP patients, the bulge rate was similar between the mesh and nonmesh groups (0 vs. 4.4%; p > 0.05); however, in bilateral DIEP patients, the bulge rate was lower in the mesh group compared with a nonmesh group (0 vs. 5.5%; p = 0.008). Conclusion Reinforcement of the anterior rectus with an onlay monofilament poly-4-hydroxybutyrate biosynthetic mesh may reduce the risk of postoperative bulge rate in patients undergoing DIEP reconstruction.

Mutation of perinatal myosin heavy chain associated with a Carney complex variant.

BACKGROUND: Familial cardiac myxomas occur in the hereditary syndrome Carney complex. Although PRKAR1A mutations can cause the Carney complex, the disorder is genetically heterogeneous. To identify the cause of a Carney complex variant associated with distal arthrogryposis (the trismus-pseudocamptodactyly syndrome), we performed clinical and genetic studies. METHODS: A large family with familial cardiac myxomas and the trismus-pseudocamptodactyly syndrome (Family 1) was identified and clinically evaluated along with two families with trismus and pseudocamptodactyly. Genetic linkage analyses were performed with the use of microsatellite polymorphisms to determine a locus for this Carney complex variant. Positional cloning and mutational analyses of candidate genes were performed to identify the genetic cause of disease in the family with the Carney complex as well as in the families with the trismus-pseudocamptodactyly syndrome. RESULTS: Clinical evaluations demonstrated that the Carney complex cosegregated with the trismus-pseudocamptodactyly syndrome in Family 1, and genetic analyses demonstrated linkage of the disease to chromosome 17p12-p13.1 (maximum multipoint lod score, 4.39). Sequence analysis revealed a missense mutation (Arg674Gln) in the perinatal myosin heavy-chain gene (MYH8). The same mutation was also found in the two families with the trismus-pseudocamptodactyly syndrome. Arg674 is highly conserved evolutionarily, localizes to the actin-binding domain of the perinatal myosin head, and is close to the ATP-binding site. We identified nonsynonymous MYH8 polymorphisms in patients with cardiac myxoma syndromes but without arthrogryposis. CONCLUSIONS: We describe a novel heart-hand syndrome involving familial cardiac myxomas and distal arthrogryposis and demonstrate that these disorders are caused by a founder mutation in the MYH8 gene. Our findings demonstrate novel roles for perinatal myosin in both the development of skeletal muscle and cardiac tumorigenesis.

Hidradenitis suppurativa complicated by squamous cell carcinoma and paraneoplastic neuropathy.

Squamous cell carcinoma is a rare complication of chronic, severe, hidradenitis suppurativa. We describe a patient with a 20-year history of extensive perineal hidradenitis suppurativa who presented with subacute muscle weakness and sensory symptoms. He was subsequently diagnosed with squamous cell carcinoma arising in the area of hidradenitis. The neurologic symptoms and signs resolved after complete excision of the tumor. This presentation is consistent with a paraneoplastic neuropathy, which has not been reported previously in patients with squamous cell carcinoma arising in patients with hidradenitis suppurativa.

Surgical correction of trismus in a child with Hecht syndrome.

Hecht syndrome is a rare condition that is also known as trismus pseudocamptodactyly syndrome. Short muscle and tendon units limiting the range of motion of upper and lower extremities and mouth characterize this disorder of muscle development. There is no consensus on the optimal treatment of temporomandibular joint (TMJ) ankylosis in this patient population. Endoscopic release in one patient resulted in early postoperative recurrence. The authors present a 28-month old boy who had a limited mouth opening of 6 mm. CT scan showed no bony ankylosis. The range of mouth opening did not to improve with physical therapy. The patient underwent extensive subperiosteal dissection of the mandible, bilateral coronoidectomy, and TMJ exploration. An intraoperative opening of 18 mm was achieved. The patient remained intubated until postoperative swelling resolved. He was extubated in the operating room 6 days later. The patient continued to improve with physical therapy. He had a 25-mm mouth opening at 12 months of follow-up.